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KMID : 0352819960110010095
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Kosin Medical Journal
1996 Volume.11 No. 1 p.95 ~ p.102
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A Clinical Study on Acute Intermittent Porphyria
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Abstract
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Acute intermittent porphyria(AIP) is a rare inborn error of metabolism characterized biochemically by the hyperexcretion of the porphyrin precursors -aminolevulinic acid(ALA) and porphobilinogen(PBG) in the urine, and clinically by
wedespread
lesions in the nervous system.
The clinical manifestations were observed in 17 patients who were diagnosed as AIP at Kosin University Gospel Hospital and Keimyung University Dongsan Hospital between Jan 1988 and Dec 1995.
@ES The results were summarized as follow:
@EN 1) The age at onset were 10-59 year old (mean 25.8±14.1 year old). The prevalence in female is higher.
2) The family history was observed in 11.8% of cases.
3) The most common precipitating factor was upper respiratory tract infection.
4) Majority of patients showed abdominal pain, tachycardia, fever, hypertension, constipation, nausea and vomiting.
5) Conventional laboratory findings were nonspecific.
6) The prognosis was good to excellent in the properly treated cases with intravenous administration of glucose.
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KEYWORD
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